Paediatric aneurysmal bone cysts of the head and neck.

OBJECTIVE: To provide an up to date review of the literature on aneurysmal bone cysts, including their diagnosis, pathology, pathophysiology, radiology and management. METHOD: Retrospective review of six cases over a 15-year period. RESULTS: Six patients (age range, eight months to 17 years; mean, 9.6 years) presented with an aneurysmal bone cyst in the mandible (n = 3), maxilla (n = 2) or occipital soft tissue (n = 1). Each patient underwent primary excision, with one subsequent recurrence. CONCLUSION: Aneurysmal bone cysts are benign but locally destructive entities which may occasionally present to otolaryngologists, since they can involve the head and neck region, in particular the mandible.

Is parental perception an accurate predictor of childhood hearing loss? A prospective study.

OBJECTIVE: We performed this study to assess whether parental perception of hearing loss predicted the results of audiometric testing in children with recurrent otitis media or persistent otitis media with effusion. METHODS: As part of a larger prospective observational outcomes study of children undergoing tympanostomy tube placement, the child's parent completed a standardized questionnaire before and after tube placement. In addition, patients underwent age-appropriate audiologic threshold and tympanometry testing before and after tube placement. RESULTS: We enrolled 113 patients (median age, 2 years), and 93 (82%) completed follow-up. Before treatment, parental perception of hearing loss did not predict hearing threshold level. After tube placement, parental perception of hearing loss was also poorly correlated with change in threshold. CONCLUSIONS: Parental perception of their child's hearing loss is a poor predictor of objective audiologic findings both before and after treatment with tympanostomy tubes. These findings have important implications concerning the importance of screening for hearing loss in children with otitis media with effusion.

Establishing the etiology of childhood hearing loss.

The cause of hearing loss in children is often difficult to identify. We evaluated a cohort of 114 children (47 boys, 67 girls) referred with newly diagnosed hearing loss (non-otitis media) to identify factors predictive of etiology and type of hearing loss. Clinical (history and physical examination), laboratory, and radiographic data were collected. One hundred children (87.7%) had sensorineural hearing loss, and 14 (12.3%) had conductive or mixed hearing loss. The cause of hearing loss was identified in 54 children (48%). Patients with isolated aural atresia (n = 7) or with a known diagnosis of congenital cytomegalovirus infection (n = 21) were excluded from further data analysis. We conducted statistical analysis to identify factors predictive of the cause and type of hearing loss. Clinical factors that aided in identifying a cause included abnormal physical examination findings (p = 0.001) and craniofacial anomalies (p = 0.006). Computed tomography of the temporal bones was the only diagnostic test predictive of cause (p < 0.001). Factors predictive of the type of hearing loss detected (sensorineural vs. conductive or mixed) were abnormal physical examination findings (p = 0.01) and craniofacial anomalies (p = 0.004). An exhaustive laboratory or radiographic workup did not prove beneficial in identifying the etiology of hearing loss in our series.

Otologic management in children with the CHARGE association.

OBJECTIVES: To characterize otologic management of two patient groups, those with the CHARGE association and those not strictly labeled as CHARGE but with several features of the disorder (CHARGE-like), in order to determine: (1) the clinical validity and utility of managing CHARGE-like children in a similar manner to patients with the strictly defined CHARGE association, (2) the progression and prognosis of hearing loss and (3) the identification of factors that may predict the degree of hearing loss. DESIGN: Case series. SETTING: Tertiary care urban children's hospital. PATIENTS: 37 children, 22 in the CHARGE group and 15 in the CHARGE-like group. INTERVENTIONS: Otorhinolaryngologic and audiologic management. MAIN OUTCOME MEASURES: Otorhinolaryngologic and audiologic evaluation. RESULTS: All patients required otologic and/or audiologic care. Bilateral hearing loss was found in 32 patients (86%) and unilateral hearing loss in five patients (14%) when hearing was assessed in the absence of otitis media. Among the 32 patients with bilateral hearing loss, 31 (97%) were able to be fit with useful hearing aids. External ear anomalies were present in 25/37 (68%) patients, and middle ear and ossicular anomalies were identified in four cases (4/37, 11%), 36/37 (97%) patients required surgical management of otitis media. Three patients (3/37, 8%) exhibited radiographic evidence of inner ear deformity. Facial nerve dysfunction was noted in the records of 14/37 (38%) patients. No statistically significant difference was found when CHARGE and CHARGE-like patients were compared for degree of hearing loss (P = 0.5964), type of hearing loss (P = 0.2657), worsening of hearing level (P = 0.7908), or anomalies of the external ear (P = 0.6921), ossicles (P = 0.7908), inner ear (P = 0.7908) or facial nerve (P = 0.6409). Patients with external ear anomalies did not exhibit statistically different degrees (P = 0.3125) or types (P = 0.1515) of hearing loss from patients without auricular anomalies. The presence of facial nerve anomaly correlated significantly (P = 0.0021) with profound hearing loss. CONCLUSIONS: Children who are CHARGE-like may be may be considered equivalent in terms of otologic and audiologic management to children strictly defined as CHARGE patients. These children all require otologic care due to the high prevalence of middle ear disease and the underlying permanent hearing loss that is both stable and aidable. The degree of hearing loss cannot be predicted by external ear morphology, but may be predicted by facial nerve palsy.

The effects of an outpatient practice guideline at a teaching hospital: a prospective pilot study.

Practice guidelines (PGs) are becoming increasingly important in modern medicine. To study the effects of a PG, we performed a pilot study at a large, urban, public teaching hospital according to a prospective, observational research design with both concurrent and historic controls. Specifically, we studied the effects of a multidisciplinary PG for pediatric outpatient tonsillectomy and adenoidectomy on the process of health-care delivery. Variables in the health-care process included patient compliance with clinic and surgery appointments, surgery time, operating room turnover, time in recovery room, unplanned admission rate, patient compliance with postoperative follow-up, provider compliance with guidelines, and hospital charges. Patients in the PG were found to have fewer preoperative laboratory tests, decreased duplication of services, and shorter operating room turnover times. Provider compliance with the PG varied by service and was intermittent at first but improved gradually. There was a trend toward improved compliance with postoperative follow-up in patients in the PG. Provider opinions concerning the guideline were positive. This pilot study demonstrates several advantages and disadvantages of the use of PGs in the outpatient setting and in a teaching hospital.

Use of an Internet-based journal club.

The on-line journal club is a useful example of the educational advantages created by the combination of current Internet technology with traditional journal club methods.

Otolaryngologic disease progression in children with human immunodeficiency virus infection.

OBJECTIVES: To evaluate the prevalence of otolaryngologic disease in children born to mothers infected with human immunodeficiency virus (HIV) and to assess the correlation between HIV disease severity and the incidence density of recurrent otitis media (OM) and sinusitis based on the revised 1994 Centers for Disease Control and Prevention (CDC) clinical-severity index. DESIGN: Case series. SETTING: Academic, tertiary care children's hospital. PATIENTS: One hundred forty-five children (73 boys, 72 girls) with vertically acquired HIV infection and 153 (77 boys, 76 girls) children who had maternal exposure to HIV but later were found not to be infected ("seroreverters"), followed up on a regular basis since birth. MAIN OUTCOME MEASURES: Prevalence of recurrent OM (3 episodes in 6 months or 4 episodes in 12 months), sinusitis, parotitis, and lymphadenopathy; incidence density of recurrent OM and sinusitis based on the 1994 CDC clinical-severity index. RESULTS: Sixty-four HIV-infected children (44%) and 13 seroreverters (8.5%) had recurrent OM (P < .001); 29 HIV-infected children (20%) and 1 seroreverter (0.6%) had sinusitis (P < .001). Eight HIV-infected patients developed tympanic membrane perforations and 25 HIV-infected children required otologic surgery. Three HIV-infected patients had parotitis. The incidence density of recurrent OM increased as HIV clinical (P = .001) and immunologic (P = .03) status worsened. In contrast, the incidence density of sinusitis did not significantly correlate with increased HIV disease severity. CONCLUSION: The prevalence of recurrent OM and sinusitis is significantly greater in HIV-infected children than in seroreverters. The incidence density of recurrent OM also significantly correlates with disease progression in HIV-infected children as measured by the 1994 CDC clinical-severity index.

Venous malformation of the pediatric airway.

OBJECTIVE: To evaluate the management of venous malformations of the pediatric airway, including diagnostic criteria and response to sclerotherapy and laser photocoagulation. DESIGN: Case series analysis of eight patients diagnosed with venous malformations of the upper airway. SETTING: Academic, tertiary care, children's hospital. PATIENTS: Eight patients (3 female, 5 male) with congenital venous malformations of the upper airway. INTERVENTION: Each patient underwent an individualized treatment regimen of sclerotherapy, laser photocoagulation and/or surgery. RESULTS: The combination of sclerotherapy and laser photocoagulation appears to cause gradual regression of the malformation. Five of the eight patients will require further therapy; two patients remain tracheotomy dependent. CONCLUSIONS: Extensive venous malformations of the pediatric airway require staged therapy, with a combination of sclerotherapy and photocoagulation. Long-term follow-up is essential because of the natural history of these lesions.

Use of intraoperative corticosteroids in pediatric tonsillectomy.

OBJECTIVE: To determine the effects of a single dose of dexamethasone sodium phosphate on postoperative morbidity in children undergoing tonsillectomy. DESIGN: Randomized, double-blind, placebo-control clinical trial. SETTING: Academic, tertiary care children's hospital. PATIENTS: Sixty-nine children (35 boys, 34 girls), aged 3 to 18 years, undergoing tonsillectomy with or without adenoidectomy. INTERVENTION: Patients were randomized to receive a single dose of intravenous dexamethasone or saline. MAIN OUTCOME MEASURES: Pain scores, determined by a Faces Scale, were obtained at 4-hour intervals during the first postoperative day and daily for 7 days thereafter. Total use of an analgesic; type of diet; level of activity; presence of halitosis, nausea, emesis, or fever; and incidence of postoperative bleeding were also compared between the two groups. RESULTS: The two groups of children were similar in age, gender, diagnosis, and surgical time. Pain scores in the postoperative period were identical while the patients were in the hospital and for the first 7 days after discharge. No statistically significant differences were noted in pain scores, nausea, emesis, halitosis, analgesic medications required, diet, or activity levels between the two groups of patients. CONCLUSION: A single intraoperative dose of dexamethasone did not appreciably affect postoperative morbidity in children undergoing tonsillectomy.

A double-blind evaluation of ketorolac tromethamine versus acetaminophen in pediatric tonsillectomy: analgesia and bleeding.

The study was designed to compare intravenous ketorolac to rectal acetaminophen for analgesia and bleeding in pediatric patients undergoing tonsillectomy. We studied 50 patients, aged 2-15 yr undergoing tonsillectomy with or without adenoidectomy. In a randomized, prospective double-blind fashion, patients were assigned to receive either ketorolac (1 mg/kg) or rectal acetaminophen (35 mg/kg). Bleeding was evaluated by measuring intraoperative blood loss and noting extra measures required to obtain hemostasis. Bleeding times were also measured before and during surgery. Pain was evaluated using a standard objective pain score for the first 3 h. Persistent pain was treated with morphine, acetaminophen, and codeine and recorded for 24 h. Blood for determination of acetaminophen levels was drawn at 20 and 40 min after the administration of study drugs. Pain scores were not significantly different between the ketorolac and acetaminophen groups. The majority of patients in both groups required additional opioid in the postoperative period. Acetaminophen levels were all less than the therapeutic range. Intraoperative bleeding times were normal in all patients, but blood loss was significantly higher in the ketorolac group (2.67 mL/kg) compared to the acetaminophen group (1.44 mL/kg), P = 0.025. Significantly more measures to achieve hemostasis were required in the ketorolac group (P = 0.012). We conclude that ketorolac is no more effective than high-dose rectal acetaminophen for analgesia in the patient undergoing tonsillectomy. Hemostasis during tonsillectomy was significantly more difficult to achieve in patients receiving ketorolac.

Malignant laryngeal tumors in children: a 15-year experience with four patients.

Malignant laryngeal neoplasms in children are uncommon and present diagnostic and therapeutic challenges. A 15-year retrospective review of laryngeal tumors at our institution identified four patients with malignant neoplasms. These children, 7 months to 16 years of age, were treated for squamous cell carcinoma, choriocarcinoma, rhabdomyosarcoma, and primitive neuroectodermal tumor. This report details these cases, emphasizing diagnostic approaches, treatment decisions (medical oncologic management, surgical resection), and rehabilitation measures for postlaryngectomy communication. A multi-institutional pediatric head and neck tumor registry would be a useful reference to assist with individual treatment decisions.

Interferon alfa-2a therapy for airway hemangiomas.

Hemangioma is a well-recognized cause of airway obstruction in the infant with stridor. Corticosteroid and laser therapy are effective in stabilizing the airway in most cases. There are, however, some extensive airway lesions that are not adequately managed by these modalities. This report describes the use of recombinant interferon alfa-2a in 15 patients with life-threatening airway hemangiomas. All patients had failed corticosteroid and/or laser therapy. Multiple upper airway sites were involved, including the base of the tongue, supraglottis, subglottis, trachea, and mediastinum. Eleven patients have completed therapy and are doing well. Four patients have resolving lesions on the drug regimen. Life-threatening airway lesions unresponsive to conventional treatment should be considered for a trial of interferon alfa-2a.

Acoustic-distortion products: separation of sensory from neural dysfunction in sensorineural hearing loss in human beings and rabbits.

Because distortion-product otoacoustic emissions (DPOAEs) provide a noninvasive measure of outer hair-cell (OHC) activity, they should provide a unique and sensitive indicator of the effects of agents that damage hearing. Using DPOAE methods, the present study was designed to assess the relative contributions of the cochlea's outer hair cells to some common sensorineural diseases, including Meniere's disease, acoustic neuroma, and noise-induced, hereditary, and sudden idiopathic sensorineural hearing loss. Parallel evaluations of DPOAEs were performed under essentially identical conditions in rabbit models of several of the human disorders, including noise-induced hearing loss, endolymphatic hydrops, and cochlear neurectomy. Animal studies were performed to assess the proficiency of DPOAEs to track a developing sensorineural deficit as well as to compare patterns of DPOAE dysfunction between clinical and experimental forms of peripheral hearing loss. Detailed measures of DPOAEs were collected in the stimulus-frequency and intensity domains as "audiograms" and response/growth or input/output functions, respectively. The outcome of analyses of both human beings and animals supported the notion that DPOAE testing is sensitive to sensory-cell disease. Thus, in combination with conventional audiometry, DPOAE measures permit a distinction between the relative contribution sensory and neural components of the cochlea make to hearing deficits.

The clinical application of acoustic distortion products.

Otoacoustic emissions provide an objective measure of hair-cell function that is independent of retrocochlear activity. Because of their frequency specificity, distortion-product emissions have great potential for clinical use. The present report describes the results of initial studies in patients with known cochlear or retrocochlear disease, diagnosed with standard otologic and audiologic tests. The cochlear group consisted of those diagnosed with noise-induced hearing loss, Meniere's disease, or hereditary hearing loss, whereas patients with acoustic neuroma comprised a retrocochlear group. A final group consisted of patients with sudden sensorineural hearing loss of unknown origin. Detailed distortion-product emission testing included the computer-controlled acquisition of "audiograms" and response/growth functions. The outcomes of these studies demonstrated that distortion-product emissions provide a noninvasive, frequency-specific test of sensory-cell function that objectively depicts the boundary between normal and abnormal hearing. These emissions were also able to effectively track dynamic changes in progressive disease processes. Finally, as a screening tool, acoustic distortion products provide an objective means of assessing cochlear status in infants and young children and in adults at risk for hearing loss. Because acoustic distortion products accurately measure the sensory component of a sensorineural hearing loss, distortion-product testing may play an important role in the diagnosis and treatment of cochlear dysfunction.

Recurrent meningitis and Mondini dysplasia.

Three infants with cerebrospinal fluid otorrhea and recurrent meningitis due to Mondini dysplasia have been treated at Baylor College of Medicine, Houston, Tex; 39 other patients with this association have been described. This review highlights the characteristics and embryologic basis of the malformation. The clinical evaluation of an infant with recurrent meningitis is outlined, and the importance of brain-stem response audiometry and thin-cut computed tomography of the temporal bones is discussed. Surgical management depends on the functional status of the dysplastic ear; usually, a stapedectomy is performed and the vestibule is obliterated. Although the surgical failure rate is 30% after one procedure, early identification of the dysplasia and prompt surgical management diminish morbidity and mortality and permit early habilitation of the child with congenital hearing impairment.

Distortion product emissions in humans. III. Influence of sensorineural hearing loss.

The realization that otoacoustic emissions are sensitive to cochlear disorders has resulted in the speculation that they may have considerable clinical potential as objective measures of hearing. To assess the clinical utility of one type of emission, the distortion product emission (DPE), a study was undertaken in individuals with hearing impairments representing a number of common otologic disorders. The results of this investigation provided evidence that tests of DPEs promise to satisfy a number of requirements important to clinical testing, including objectivity of measurement procedures, test-retest reliability, simple subject preparation, readily available instrumentation, and relatively brief examination periods. The fine resolution of DPEs within the stimulus frequency and level domains also permits an accurate confirmation of the pattern of hearing loss. For example, tests of DPEs detected a 20-dB hearing level impairment at a single frequency in an ear exhibiting early signs of noise-induced hearing loss, and a 10-dB improvement in sensitivity following ingestion of the hyperosmotic agent glycerol in an ear displaying a mild to moderate hearing loss due to Meniere's disease. Finally, the application of DPEs to the objective testing of otologic disorders suggests that the ability of these responses to assess the sensory component of a sensorineural disorder may contribute to the eventual understanding of the complicated pathogenesis of many cochlear diseases. When all the positive features of DPE testing are realized, the potential contribution that these measures can make in a clinical setting becomes apparent.

Hemoperitoneum from perforated Meckel's diverticulum.

Approximately 4% of patients with Meckel's diverticulum will experience complication, the most common of which are intestinal obstruction, gastrointestinal bleeding, acute inflammation, and perforation. We report an extremely rare complication of perforated Meckel's diverticulum which presented as hemoperitoneum.